Benign esophageal neoplasms are rare, and malignant neoplasms make up the vast majority of all esophageal neoplasms. For this reason, though they account for approximately 6% of all gastrointestinal tract cancers, they are responsible for a disproportionately large number of deaths. The commonest benign neoplasm of the esophagus is a leiomyoma, a tumor of smooth muscle cells. Virtually all malignant neoplasms of the esophagus are epithelial in origin (carcinomas), either squamous cell carcinomas (SCC) or adenocarcinomas.
Adenocarcinoma is preceded by Barrett's esophagus in virtually all cases, and arises through a well-defined sequence as follows: long-standing gastroesophageal reflux disease, intestinal metaplasia (Barrett's esophagus), low grade dysplasia, high grade dysplasia, then adenocarcinoma.
About half of all esophageal squamous cell carcinoma cases arise in the lower third of the esophagus, with the remaining cases arising in the upper and middle thirds. Tumors may be polypoid, ulcerating or infiltrating. Histologically, squamous differentiation is evident with the presence of keratin pearls in better differentiated examples.
Virtually all cases of adenocarcinoma arise in the distal esophagus in association with Barrett's esophagus. These tumors are more often ulcerating or infiltrating than polypoid, and histologically show evidence of glandular differentiation.
Both neoplasms spread directly through the wall into adjacent structures (easy because of the lack of serosa) or via the lymphatics or venous drainage.
Dysphagia is a frequent presenting symptom in patients with esophageal carcinoma. Other symptoms include anorexia, cachexia, pain, voice hoarseness if the recurrent laryngeal nerve is involved, and cough if there is a tracheoesophageal fistula.
Adenocarcinomas are typically associated with a long history of reflux symptoms because of the links to gastroesophageal reflux disease. Treatment options are primarily surgical, though patients must be carefully selected because esophagectomy carries a high mortality rate in its own right. Radiation therapy and chemotherapy unfortunately play limited roles.
The prognosis may be assessed with clinical and pathological staging. The 5-year survival of patients with superficial cancers is ~75% as opposed to ~10-25% for patients with advanced (deeply invasive) cancers who undergo curative resection.